CASE OF THE MONTH
A painful, red eye Karen Kushner, PA-C; Jeffrey Kushner, DO
CASE A 25-year-old woman presented to the ED with progressively worsening left eye pain and swelling for 2 days. She also complained of tearing and redness of the left eye as well as painful extraocular movements. She denied vision changes, although she had trouble opening her left eye due to swelling. Her right eye was unaffected. Additionally, she denied trauma, sick contacts, sinus pain, recent dental procedures, headache, or fever. History The patient’s past medical history was only significant for asthma. She had no personal or family history of any coagulopathic disorders. She does not wear contacts or glasses, takes no medications, and denied use of recreational drugs. Physical examination The patient was alert and oriented, and in moderate distress. She was afebrile and her vital signs were: BP, 133/79 mm Hg; heart rate, 71; respirations, 18; temperature, 98.9° F (37.2° C); Spo2, 98% on room air. Her left eye was injected with marked periorbital swelling and mild erythema. Pupils were equal and reactive. She had painful extraocular movements of the left eye and some clear tearing from the affected eye. Her anterior chamber was clear with an otherwise unremarkable fundoscopic exam. She had no proptosis or sinus tenderness. Her cranial nerves were intact. She had a slight decrease in visual acuity on the left. Her ears, nose, and oropharynx were unremarkable. She had full range of motion in her neck without tenderness or bruits. A complete blood cell (CBC) count showed a white blood cell (WBC) count of 10,000 cells/mm3. Results of a CT scan of her orbits with contrast are shown in Figure 1. WHAT IS YOUR DIAGNOSIS? • orbital cellulitis • Grave disease (hyperthyroidism) • idiopathic orbital inflammatory disease • granulomatosis with polyangiitis
At Largo (Fla.) Medical Center, Karen Kushner is a PA in emergency medicine and Jeffrey Kushner is a medical resident. The authors have disclosed no potential conflicts of interest, financial or otherwise. DOI: 10.1097/01.JAA.0000455657.27295.65 Copyright © 2014 American Academy of Physician Assistants
54
www.JAAPA.com
FIGURE 1.
DISCUSSION The patient has idiopathic orbital inflammatory disease, a noninfectious, benign condition marked by inflammation of the orbit without an identifiable local or systemic cause.1 Orbital cellulitis must be considered, as a missed diagnosis can lead to local and intracranial spread with catastrophic outcomes such as blindness and death. However, patients with orbital cellulitis usually present with fever and leukocytosis and often have a history of recent sinusitis or trauma.2 Hyperthyroidism is the most common cause of exophthalmos and displays a similar constellation of ocular symptoms but usually has a more insidious onset, is often bilateral, painless, and causes a more prominent lid lag. Patients with hyperthyroidism also frequently have symptoms of heat intolerance, weight loss, tachycardia, and tremor. Granulomatosis with polyangiitis is a necrotizing, granulomatous vasculitis that also affects the respiratory and renal systems. Rhinitis is usually the first sign in most of these patients. Orbital involvement is due to an extension of sinonasal disease and often bilateral.2 Idiopathic orbital inflammatory disease was originally called orbital pseudotumor due to its resemblance to a neoplasm on imaging. The exact pathogenesis remains uncertain, but infectious causes or autoimmune processes have been postulated. After hyperthyroidism and lymphoproliferative diseases, it is the third most common orbital disease.1 The condition is most frequently seen in middle-aged patients, but affects all ages, sexes, and races. Pediatric cases account for about 17% of all cases.1 The most typical presentation is an acute, unilateral onset of pain with associated Volume 27 • Number 11 • November 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
A painful, red eye
edema, erythema, and proptosis. Subacute, chronic, and bilateral cases are less common. Any orbital structure may be affected, but involvement of the bony orbit is rare.1 Although benign, severe idiopathic orbital inflammatory disease can lead to vision loss and oculomotor dysfunction.1 All patients with suspected idiopathic orbital inflammatory disease require a full ophthalmic workup, including a visual acuity test, fundoscopic examination, and slit lamp examination. Once physical examination confirms the suspicion for idiopathic orbital inflammatory disease, obtain CT or MRI for tissue characterization and localization. CT often reveals a poorly demarcated, focal, or diffuse mass that enhances with contrast. Systemic corticosteroids remain the mainstay of treatment, although successful outcomes with cyclophosphamide, methotrexate, cyclosporine, and radiation therapy have been reported in patients unresponsive to corticosteroids.1 Biopsy can be considered in recurrent or refractory cases.
hospital and started on IV antibiotics for possible left orbital cellulitis with abscess in the left medial rectus muscle. Over the next 24 hours, her symptoms showed little improvement, so she was started on prednisone. She remained afebrile and without leukocytosis throughout her hospital stay. At the time of discharge on day 3, the patient had responded well. She was sent home with prescriptions for cephalexin and a tapering course of prednisone. Unfortunately, she returned to the ED 4 days later with the same worsening symptoms as her initial presentation. A repeat CT of the orbit showed an abnormal enlargement within the left medial rectus muscle, although slightly decreased when compared to the previous CT. Idiopathic orbital inflammatory disease was diagnosed and a longer tapering dose of prednisone prescribed. Her symptoms gradually resolved and she has been followed by ophthalmology since then. JAAPA REFERENCES
MANAGEMENT The patient’s CT showed left periorbital swelling and a small collection within the left medial rectus muscle without intraorbital fat stranding. She was admitted to the
JAAPA Journal of the American Academy of Physician Assistants
1. Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 2003;121(4):491-499. 2. Lutt JR, Lim LL, Phal PM, Rosenbaum JT. Orbital inflammatory disease. Semin Arthritis Rheum. 2008;37(4):207-222.
www.JAAPA.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
55
A painful, red eye Karen Kushner, PA-C; Jeffrey Kushner, DO
CASE A 25-year-old woman presented to the ED with progressively worsening left eye pain and swelling for 2 days. She also complained of tearing and redness of the left eye as well as painful extraocular movements. She denied vision changes, although she had trouble opening her left eye due to swelling. Her right eye was unaffected. Additionally, she denied trauma, sick contacts, sinus pain, recent dental procedures, headache, or fever. History The patient’s past medical history was only significant for asthma. She had no personal or family history of any coagulopathic disorders. She does not wear contacts or glasses, takes no medications, and denied use of recreational drugs. Physical examination The patient was alert and oriented, and in moderate distress. She was afebrile and her vital signs were: BP, 133/79 mm Hg; heart rate, 71; respirations, 18; temperature, 98.9° F (37.2° C); Spo2, 98% on room air. Her left eye was injected with marked periorbital swelling and mild erythema. Pupils were equal and reactive. She had painful extraocular movements of the left eye and some clear tearing from the affected eye. Her anterior chamber was clear with an otherwise unremarkable fundoscopic exam. She had no proptosis or sinus tenderness. Her cranial nerves were intact. She had a slight decrease in visual acuity on the left. Her ears, nose, and oropharynx were unremarkable. She had full range of motion in her neck without tenderness or bruits. A complete blood cell (CBC) count showed a white blood cell (WBC) count of 10,000 cells/mm3. Results of a CT scan of her orbits with contrast are shown in Figure 1. WHAT IS YOUR DIAGNOSIS? • orbital cellulitis • Grave disease (hyperthyroidism) • idiopathic orbital inflammatory disease • granulomatosis with polyangiitis
At Largo (Fla.) Medical Center, Karen Kushner is a PA in emergency medicine and Jeffrey Kushner is a medical resident. The authors have disclosed no potential conflicts of interest, financial or otherwise. DOI: 10.1097/01.JAA.0000455657.27295.65 Copyright © 2014 American Academy of Physician Assistants
54
www.JAAPA.com
FIGURE 1.
DISCUSSION The patient has idiopathic orbital inflammatory disease, a noninfectious, benign condition marked by inflammation of the orbit without an identifiable local or systemic cause.1 Orbital cellulitis must be considered, as a missed diagnosis can lead to local and intracranial spread with catastrophic outcomes such as blindness and death. However, patients with orbital cellulitis usually present with fever and leukocytosis and often have a history of recent sinusitis or trauma.2 Hyperthyroidism is the most common cause of exophthalmos and displays a similar constellation of ocular symptoms but usually has a more insidious onset, is often bilateral, painless, and causes a more prominent lid lag. Patients with hyperthyroidism also frequently have symptoms of heat intolerance, weight loss, tachycardia, and tremor. Granulomatosis with polyangiitis is a necrotizing, granulomatous vasculitis that also affects the respiratory and renal systems. Rhinitis is usually the first sign in most of these patients. Orbital involvement is due to an extension of sinonasal disease and often bilateral.2 Idiopathic orbital inflammatory disease was originally called orbital pseudotumor due to its resemblance to a neoplasm on imaging. The exact pathogenesis remains uncertain, but infectious causes or autoimmune processes have been postulated. After hyperthyroidism and lymphoproliferative diseases, it is the third most common orbital disease.1 The condition is most frequently seen in middle-aged patients, but affects all ages, sexes, and races. Pediatric cases account for about 17% of all cases.1 The most typical presentation is an acute, unilateral onset of pain with associated Volume 27 • Number 11 • November 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
A painful, red eye
edema, erythema, and proptosis. Subacute, chronic, and bilateral cases are less common. Any orbital structure may be affected, but involvement of the bony orbit is rare.1 Although benign, severe idiopathic orbital inflammatory disease can lead to vision loss and oculomotor dysfunction.1 All patients with suspected idiopathic orbital inflammatory disease require a full ophthalmic workup, including a visual acuity test, fundoscopic examination, and slit lamp examination. Once physical examination confirms the suspicion for idiopathic orbital inflammatory disease, obtain CT or MRI for tissue characterization and localization. CT often reveals a poorly demarcated, focal, or diffuse mass that enhances with contrast. Systemic corticosteroids remain the mainstay of treatment, although successful outcomes with cyclophosphamide, methotrexate, cyclosporine, and radiation therapy have been reported in patients unresponsive to corticosteroids.1 Biopsy can be considered in recurrent or refractory cases.
hospital and started on IV antibiotics for possible left orbital cellulitis with abscess in the left medial rectus muscle. Over the next 24 hours, her symptoms showed little improvement, so she was started on prednisone. She remained afebrile and without leukocytosis throughout her hospital stay. At the time of discharge on day 3, the patient had responded well. She was sent home with prescriptions for cephalexin and a tapering course of prednisone. Unfortunately, she returned to the ED 4 days later with the same worsening symptoms as her initial presentation. A repeat CT of the orbit showed an abnormal enlargement within the left medial rectus muscle, although slightly decreased when compared to the previous CT. Idiopathic orbital inflammatory disease was diagnosed and a longer tapering dose of prednisone prescribed. Her symptoms gradually resolved and she has been followed by ophthalmology since then. JAAPA REFERENCES
MANAGEMENT The patient’s CT showed left periorbital swelling and a small collection within the left medial rectus muscle without intraorbital fat stranding. She was admitted to the
JAAPA Journal of the American Academy of Physician Assistants
1. Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 2003;121(4):491-499. 2. Lutt JR, Lim LL, Phal PM, Rosenbaum JT. Orbital inflammatory disease. Semin Arthritis Rheum. 2008;37(4):207-222.
www.JAAPA.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
55
